Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia

Hamza, Alaa F.; Soliman, Hesham A.; Hay, Sameh Abdel; Kabesh, Ashraf A.; Elbehery, M M

doi:10.1053/jpsu.2001.27943

Cited by 27 publications

(14 citation statements)

References 7 publications

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“…A much lower incidence of vaginal stenosis was reported on using TUM, ranging from 0% [23,29] to 9.1% [24]. Similar to other studies [23,29], we reported 0% incidence of urethrovaginal fistula or hypospadic urethral meatus. We reported vaginal stenosis in two patients (14.3%) and they needed only vaginal dilatation.…”

Section: Discussionsupporting

confidence: 87%

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Feminizing genitoplasty in congenital adrenal hyperplasia

Shoeir¹,

Wali²,

AbdelHamid³

et al. 2012

Annals of Pediatric Surgery

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Section: Discussionsupporting

confidence: 87%

“…Before the era of TUM, there was a high incidence of vaginal stenosis of up to 78.5% and most patients required further vaginal reconstructive procedures [28]. A much lower incidence of vaginal stenosis was reported on using TUM, ranging from 0% [23,29] to 9.1% [24]. This is obviously because TUM decreased the incidence of vaginal ischemia.…”

Section: Discussionmentioning

confidence: 99%

Feminizing genitoplasty in congenital adrenal hyperplasia

Shoeir¹,

Wali²,

AbdelHamid³

et al. 2012

Annals of Pediatric Surgery

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“…Numerous techniques have been reported for feminizing genitoplasty during the last few years [10][11][12]. A cut-back of the UGS combined with a Fortunoff inverted U-shaped perineal flap is used in cases when the urethra and the vagina share low confluence [13].…”

Section: Discussionmentioning

confidence: 99%

Surgical aspects of ambiguous genitalia associated with congenital adrenal hyperplasia

Lamoshi¹,

El-Gharmooll²

2015

Annals of Pediatric Surgery

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Aim The aim of the study was to review the current approach to manage ambiguous genitalia caused by CAH.Methods This was a retrospective study of 30 patients with CAH and ambiguous genitalia treated over 10 years. Age at presentation, degree of verilization, preoperative diagnostic studies, operative technique, blood loss, outcome, and follow-up period were the studied variables. ResultsThe older 19 patients underwent single-stage feminizing genitoplasty and the younger 11 patients underwent a multiple-stage procedure. All patients had undergone clitoroplasty, clitoral recession, or resection. Postoperative complications documented in 23% of the patients included atrophy of the clitoris, flaps complication, and vaginal stenosis. The anatomic and cosmetic outcomes were considered good or satisfactory in 70% and 20% of cases, respectively, and poor in 10% of cases.Conclusion One-stage or multiple-stage feminizing genitoplasty gives a high percentage of satisfactory cosmetic outcomes. TUM can be implemented to achieve excellent results for complex genitourinary anomalies. Ann

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“…In the situation where supine positioning is also required, we have been able to reposition the patient with no difficulty. Similar to other centres that have used the TUM [15], the continence status is not yet available, but certainly is a concern. The use of TUM has facilitated reconstruction of the persistent cloaca.…”

Section: Discussionmentioning

confidence: 99%

Upper and lower urinary tract outcome after surgical repair of cloacal malformations: a three‐decade experience

et al. 2005

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The three papers in this section cover a wide range of subjects. Authors from Indianapolis present a three‐decade experience in upper and lower urinary tract outcomes after the surgical repair of cloacal malformations. An objective assessment of their results of hypospadias surgery is described by authors from Manchester. Finally, the question is asked by authors from Athens; is bladder muscle decompensation in boys with a history of PUV caused by secondary bladder neck obstruction? OBJECTIVE To report the urological outcome of the surgical correction of persistent cloaca, which is technically demanding and may require many procedures in an effort to preserve renal function and provide urinary continence. PATIENTS AND METHODS A retrospective chart review from 1971 to 2003 identified 23 patients with cloacal malformations (two posterior, 21 classical) that were reconstructed. The confluence of the urethra, vagina and rectum was noted to be high in 16, low in five and unknown in two; one patient was a conjoined twin. RESULTS The mean (range) follow‐up was 9.3 (0.4–31.6) years. Urinary anomalies included 14 patients with renal anomalies (six solitary kidneys, four renal dysplasia, two pelvi‐ureteric junction, one each duplex and crossed fused) and two duplicated bladders. Vesico‐ureteric reflux was present in 13 patients (57%), hydronephrosis at birth in 13, a bony vertebral abnormality in 14 and the VACTERL association in four. Total urogenital mobilization (TUM) was used in six patients and spinal cord untethering in four; a nephrectomy was required in three and partial nephrectomy in one. Upper tract dilation was still present in six patients. Age‐adjusted creatinine levels were abnormal in four (18%) patients and borderline in another six (26%). In the nine patients with a solitary kidney (six solitary, three after nephrectomy), the age‐adjusted creatinine level was abnormal in two and borderline in four. A vesicostomy was initially performed in 11 patients. The method of bladder emptying is known in 22; 10 void, 11 require clean intermittent catheterization (five abdominal stoma, six urethral) and one was diverted with a conduit. Of the 18 patients aged > 47 months 15 were continent (14 complete > 4 h, one partial 2–4 h), and three are wet (one conduit). Reconstruction of the lower urinary tract included four bladder augmentations (one ureteric, one ileal, two colon), five bladder neck procedures (two artificial sphincter, one each bladder neck repair, sling, bladder neck division) and six catheterizable channels (one now with a colon conduit). The ureters were re‐implanted in 12 patients. CONCLUSION Although the surgical correction of this rare malformation is complex, the upper urinary and lower urinary tract outcome can be favourable, albeit after several reconstructive procedures. TUM has emerged as the primary method for vaginal reconstruction, but the long‐term lower tract outcome after this procedure is awaited.

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Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia

Cited by 27 publications

References 7 publications

Feminizing genitoplasty in congenital adrenal hyperplasia

Feminizing genitoplasty in congenital adrenal hyperplasia

Surgical aspects of ambiguous genitalia associated with congenital adrenal hyperplasia

Upper and lower urinary tract outcome after surgical repair of cloacal malformations: a three‐decade experience

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